Comparative efficacy and safety of different minimal invasive pyeloplasty in treating patients with ureteropelvic junction obstruction: a network meta-analysis.

OBJECTIVE: In recent years, the minimally invasive surgical treatment methods of ureteropelvic junctional obstruction (UPJO) have been diverse, but its approach and choice of surgical method are controversial. This network meta-analysis (NMA) aimed to compare the safety and effectiveness of minimally invasive surgeries for UPJO, which included robotic or laparoscopic pyeloplasty, via the retroperitoneal or transperitoneal approach. METHODS: We searched relevant RCTs in PubMed, Embase, Web of Science, the Cochrane Library, and CNKI. To assess the results of operative time, complications and success rate, pairwise, and NMA were carried out. The models for analyses were performed by Revman 5.3, Addis V1.16.8 and R software. RESULTS: A total of 6 RCTs were included in this study involving four types of surgeries: transperitoneal laparoscopic pyeloplasty (T-LP), retroperitoneal laparoscopic pyeloplasty (R-LP), robot-assisted transperitoneal pyeloplasty (T-RALP), and robot-assisted retroperitoneal pyeloplasty (R-RALP). This study consisted of 381 patients overall. T-RALP had a quicker operational duration (SMD = 1.67, 95% CI 0.27-3.07, P = 0.02) than T-LP. According to the NMA's consistency model, T-RALP improved the surgical success rate more than T-LP (RR = 6303.19, CI 1.28 to 1.47 × 1011). Ranking probabilities indicated that RALP could be the better option than LP and retroperitoneal approach was comparable to transperitoneal approach. All procedures had high surgical success rates and few complications. CONCLUSION: Outcomes for four surgical approaches used in the UPJO were comparable, with T-RALP being the most recommended approach. Selection between the transperitoneal and retroperitoneal approaches primarily depended on the surgeon's preference. Higher quality evidence is needed to further enhance the result.

A mutual promotion encoder-decoder method for ultrasonic hydronephrosis diagnosis.

As a common cause of hydronephrosis in children, ureteropelvic junction obstruction (UPJO) may lead to a series of progressive renal dysfunction. Ultrasonography is a primary screening of UPJO, yet its further examinations are laborious, time-consuming, and mostly radioactive. The deep learning based automatic diagnosis algorithms on UPJO or hydronephrosis ultrasound images are still rare and performance remains unsatisfactory owning to limitation of manually identified region of interest, small dataset and labels from single institution. To relieve the burden of children, parents, and doctors, and avoid wasting every bit information in all datasets, we hence designed a deep learning based mutual promotion model for the auto diagnosis of UPJO. This model consists of a semantic segmentation section and a classification section, they shared a mutual usage of a transformation structure by separately training the encoder and decoder and loop this circle. Thorough comparative experiments are conducted and situations are explored by ablation experiments, results shown our methods outperformed classic networks with an accuracy of 0.891 and an F1-score of 0.895. Our design can jointly utilize different supervisions and maximize the use of all the characteristics of each dataset, and automatically diagnose the severity of UPJO on the basis of ultrasound images by first segmentate then classify the images, moreover, not only is the final result excellent, but also the midway segmentation result is also very accurate and have smooth edges that are convenient for doctors to recognize with their naked eyes. All in all, our proposed method can be an important auxiliary tool for smart healthcare.

Double J stent combined with pyelostomy tube in pediatric laparoscopic pyeloplasty: a 5-year clinical experience in a single center.

OBJECTIVE: To compare the outcome of using a double J (DJ) stent combined with pyelostomy tube with a DJ stent alone in laparoscopic pyeloplasty (LP) for pediatric ureteropelvic junction obstruction (UPJO). METHODS: A retrospective review of all patients with UPJO treated with LP between January 2017 and November 2021 was conducted in our center. According to different postoperative drainage methods patients were divided into a DJ stent group (52 cases) and a DJ stent combined with pyelostomy tube group (combination group, 41 cases). Operative time, bleeding volume, perirenal drainage stent removal time, postoperative hospital stay, postoperative complications, and renal function recovery were compared between the two groups. Renal ultrasound and diuretic renogram (DR) were used for preoperative and postoperative follow-up. RESULTS: A total of 52 patients were in the DJ stent group and 41 patients in the combination group. The mean hospital stay was 6.46 ± 2.66 days in the DJ stent group and 5.22 ± 1.63 days in the combination group (p < 0.05). Postoperative complications developed in 14 out of 52 patients in the DJ stent group (26.9%), while complications developed in 8 out of 41 patients in the combination group (19.5%) (p > 0.05). Non-catheter-related complications developed in 10/52 patients in the DJ stent group (19.2%) and only 1/41 patients in the combination group (2.4%) (p < 0.05). The renal function and renal cortex thickness in both groups were improved. CONCLUSION: Both the DJ stent drainage and the DJ stent combined with pyelostomy drainage are safe and effective. We should fully consider the patient's preoperative and intraoperative conditions and choose appropriate drainage methods. A DJ stent combined with pyelostomy tube can reduce non-catheter related complications, facilitate postoperative recovery, and the hospital stay was significantly shorter than the DJ stent group. However, it is necessary to pay attention to the nursing treatment of the pyelostomy tube and guard against the occurrence of pyelostomy tube shedding.

Comparison of early surgical and conservative therapy in children with ureteropelvic junction obstruction.

BACKGROUND: Ureteropelvic junction obstruction is a relatively common urologic problem in children. Most cases present with pelvicaliceal dilatation in antenatal period. Historically most UPJO cases were treated with surgical procedures, but recently many of these children have been treated by nonsurgical observational plans. We compared the outcome of children with UPJO treated in surgical and observational ways. METHODS: In a retrospective study, we assessed the medical history of patients diagnosed as UPJO, march 2011 to march 2021. The case definition was based on grade 3-4 hydronephrosis and obstructive pattern in dynamic renal isotopes can. Patients were put into two groups; Group 1 children were treated with a surgical procedure, and group 2 patients without any surgical procedure for at least a six months' period after diagnosis. We assessed long-term events and improvement of obstruction. RESULTS: Seventy-eight children (mean age 7.32mo., 80% male) enrolled in the study, 55 patients in group one and 23 as group 2. Severe hydronephrosis was the problem of 96% of all patients significantly led to 20% in group 1 and 9% in group 2 (P < 0.001). Severe kidney involvement was observed at 91% in group 1 and 83% in group 2, decreased to 15% and 6%, respectively (P < 0.001). There were no significant differences in sonographic and functional improvement between the two intervention groups. Long-term prognostic issues; growth, functional impairment, and hypertension were not different between the two groups, but group 1 children experienced more recurrence of UTI than group 2 patients. CONCLUSION: Conservative management is as effective as early surgical treatment in the management of infants with severe UPJO.

Sonographic follow-up after pyeloplasty: a large, retrospective cohort analysis.

PURPOSE: Routine scintigraphy after surgery for uretero-pelvic junction obstruction (UPJO) is discouraged, making ultrasound the preferred option for follow up. Yet, interpretation of sonographic parameters is rarely straightforward. METHODS: We reviewed 111 cases including 97 pyeloplasty (52 open, 45 laparoscopic) and 14 pyelopexy during a 7-year period. Pre- and postoperative pelvic antero-posterior diameter (APD), cortical thickness (CT) and pelvis/cortex ratio (PCR) was measured serially. RESULTS: 85% were free of symptoms by 1 year. Only 11% had complete resolution of hydronephrosis. Eleven (10.4%) needed a redo procedure. Mean reduction in APD was 32.6%, 45.8%, and 51.7% at 6 weeks, 3 and 6 months respectively. CT increased by an average 55.9%, 75.6% and 107.6% while PCR reduced by 6.9, 8.0 and 8.8 at given intervals. Comparison of open and laparoscopic procedures showed no significant difference. Review of failed pyeloplasty showed failure of reduction in APD (APD > 3 cm or < 25% reduction) and PCR (PCR > 4) as early indicators for failure. CONCLUSION: Both APD and PCR are reliable indicators of success and failure following pyeloplasty while CT alone is not as useful. Laparoscopic procedures are non-inferior to standard open surgery.

A Novel nonsense variant in the CDH2 gene associated with ACOGS: A case report.

Agenesis of Corpus Callosum, Cardiac, Ocular, and Genital Syndrome (ACOGS; OMIM #618929) is a rare genetic disorder characterized by global developmental delay, agenesis or hypoplasia of corpus callosum, craniofacial dysmorphism, ocular, cardiac, and genital anomalies. ACOGS is caused by variations in the CDH2 gene. Our patient had a novel finding besides the classical findings of ACOGS. To the best of our knowledge, only 14 patients with ACOGS have been reported. Here, we reported the fifteenth patient with ACOGS, having a novel de novo nonsense variant in the CDH2 gene, and the first patient from Turkey with a novel finding. Our patient was the first female to have a renal anomaly since only genital malformations were reported in male patients (cryptorchidism, micropenis) so far.

Laparoscopic and robotic pyeloplasty as minimally invasive alternatives to the open approach for the treatment of uretero-pelvic junction obstruction in infants: a multi-institutional comparison of outcomes and learning curves.

BACKGROUND: Since the development of minimally invasive surgery (MIS), laparoscopic and robotic approaches have been widely adopted. However, little has been published detailing the learning curve of MIS, especially in infants. OBJECTIVE: To quantify the learning curve of laparoscopic (LP) and robot-assisted laparoscopic pyeloplasty (RAL-P) for treatment of uretero-pelvic junction obstruction (UPJO) in infants evidenced by number of cases, operative time, success and complications. PATIENTS AND METHODS: Between 2009 and 2017, we retrospectively reviewed pyeloplasty cases for treatment of UPJO in infants at three academic institutions. The primary outcome was success. Secondary outcomes were UPJO recurrence, complications, and operative time as a surrogate of skill acquisition. Continuous variables were analyzed by t test, Welch-test, and one-way ANOVA. Non-continuous variables were analyzed by Chi-squared test or Fisher's exact test. Learning curves (LC) were studied by r-to-z transformation and CUSUM. RESULTS: Thirty-nine OP, 26 LP, and 39 RAL-P had mean operative times (OT) of 106, 121, and 151 min, respectively. LCs showed plateau in OT after 18 and 13 cases for LP and RAL-P, respectively. RAL-P showed a second phase of further improvements after 37 cases. At 16 months follow-up, there were similar rates of success and complications between the three groups. CONCLUSIONS: Despite different duration of learning phases, proficiency was achieved in both LP and RAL-P as evidenced by stabilization of operative time and similar success rates and complications to OP. Before and after achievement of proficiency, LP and RAL-P can be safely learned and implemented for treatment of UPJO in infants.

Ability of volume measures of hydronephrosis to predict need for surgery and evaluate renal function in children with ureteropelvic junction obstruction.

OBJECTIVE: To explore the efficacy of quantitative renal volume measures on magnetic resonance urography images in predicting need for surgery among children with ureteropelvic junction obstruction and their ability to evaluate renal function. METHODS: A total of 88 cases of hydronephrosis in 50 patients were collected between 1 April 2018 and 31 March 2020, including 30 operated kidney and 58 unoperated kidney cases. Clinical data were collected, and quantitative analysis of magnetic resonance urography was performed. Renal volume, hydronephrosis volume and the volume ratio of hydronephrosis (hydronephrosis volume/renal volume) were measured and calculated. We analyzed the relationships between the above indices in the two groups and compared these with renal function. RESULTS: Compared with the unoperated kidney group, hydronephrosis volume, renal volume and hydronephrosis volume/renal volume of the operated kidney group increased significantly. Hydronephrosis volume (area under the curve 0.972, 95% confidence interval 0.943-1.000; P < 0.001) and hydronephrosis volume/renal volume (area under the curve 0.968, 95% confidence interval 0.939-0.998; P < 0.001) were superior to ultrasonography and renal function examination in predicting the probability of surgery, and their sensitivity values (hydronephrosis volume/renal volume: 96.67%; hydronephrosis volume: 93.33%) were higher than those of the renal function test (50%). There was a significant difference among different renal function groups in the pairwise comparison of hydronephrosis volume and hydronephrosis volume/renal volume (P < 0.05). CONCLUSION: Quantitative volume measures of hydronephrosis by magnetic resonance urography had a greater ability to predict need for surgery than ultrasonography and dynamic renal imaging, and it can be used as method by which to evaluate surgery. Hydronephrosis volume and hydronephrosis volume/renal volume have greater predictive ability, and play an important role in the deterioration of renal function.

Comparison of continuous and interrupted suture techniques in pyeloplasty: a systematic review and meta-analysis.

Pelvi-ureteric anastomosis is a critical step to ensure good outcome of pyeloplasty. Continuous suturing technique, especially for laparoscopic surgeries, may offer faster operative time while allowing water-tight anastomosis and remains an alternative to interrupted suturing technique. There has been mixed data on comparison of outcomes of continuous and interrupted suturing techniques. This systematic review and meta-analysis aim to assess the outcomes of pyeloplasty based on continuous and interrupted suturing techniques. Following protocol registration on PROSPERO (CRD42021269706), a systematic review was performed in accordance with Cochrane Collaboration. A literature search was performed in September 2021 across Medline, EMBASE, Scopus, Cochrane Library, and ClinicalTrials.gov. Records comparing pyeloplasty outcomes between continuous and interrupted suture techniques were included. Five studies were identified for inclusion (2 prospective, 3 retrospective). Three studies involved pediatric patients. Three studies exclusively assessed laparoscopic technique. Four outcomes were meta-analyzed: operative time, length of stay, complications, and pyeloplasty failure. Interrupted sutures had longer OR time (mean difference 33.14 min [95% CI 29.35-36.94], p < 0.0001) and length of stay (mean difference 1.08 days [95% CI 0.84-1.32], p < 0.0001). However, there were similar complication (OR 1.73 [95% CI 0.98-3.06], p = 0.06) and failure rates (OR 1.21 [95% CI 0.43-3.43], p = 0.71) between the two suture types. The overall risk of bias in the studies was high. While limited by the number of studies available, continuous sutures for pelvi-ureteric anastomosis appear to confer benefits of faster operative time and decreased length of stay without increasing complication rates or failures.

Mini-laparoscopic pyeloplasty to treat UPJO in infants.

PURPOSE: The aim of this study was to investigate the safety and clinical efficacy of mini-laparoscopic pyeloplasty in treating ureteropelvic junction obstruction (UPJO) in infants. MATERIAL AND METHODS: We retrospectively analysed the clinical data of 66 infants with UPJO from January 2013 to August 2018 at our hospital. They were divided into the laparoscopic surgery group (group A) and the open surgery group (group B), depending on the surgical method. RESULTS: The bleeding volume, analgesia duration, postoperative hospitalization duration, and incision length in group A were significantly less than those in group B (p < .05). The incidence of incision dehiscence was 0% in group A and 11.7% in group B (p = .045). At the postoperative follow-up, the incidence of anastomotic stenosis was 6.2% in group A and 5.9% in group B (p = .719). The anteroposterior diameter and glomerular filtration rate were significantly improved at the one-year follow-up, but there was no significant difference between the groups (p > .05). CONCLUSIONS: Mini-laparoscopic pyeloplasty to treat UPJO in infants has the same early clinical efficacy and safety as open surgery, and this procedure has the advantages of fewer incisions, less pain, quicker recovery, and better cosmetic outcomes.

Serum-Targeted HILIC-MS Metabolomics-Based Analysis in Infants with Ureteropelvic Junction Obstruction.

Ureteropelvic junction obstruction (UPJO) constitutes the predominant cause of obstructive nephropathy in both neonates and infants. Fundamental questions regarding UPJO's mechanism, assessment, and treatment still remain unanswered. The aim of the present study was to elucidate potential differences through serum metabolic profiling of surgical cases of infants with UPJO compared to both nonsurgical cases and healthy age-matched controls. Early diagnosis of renal dysfunction in this cohort based on highlighted biomarkers was the ultimate goal. Thus, serum samples were collected from 20 patients preoperatively, 19 patients with mild stenosis treated conservatively, and 17 healthy controls. All samples were subjected to targeted metabolomics analysis by hydrophilic interaction liquid chromatography coupled to mass spectrometry (HILIC LC-MS/MS). Both univariate and multivariate statistical analyses were performed. Principal component analysis (PCA) and orthogonal partial least squares-discriminant analysis (OPLS-DA) score plots showed that the studied groups differed significantly, with a panel of metabolites, including creatinine, tryptophan, choline, and aspartate, distinguishing patients who required surgery from those followed by systematical monitoring as well as from healthy controls, showing high performance as indicators of UPJO disease.

MMP9 overexpression is associated with good surgical outcome in children with UPJO: Preliminary results.

BACKGROUND: Ureteropelvic junction obstruction (UPJO) diagnosed prenatally occurs in 1:150 - 1:1200 pregnancies. Although many studies investigating the molecular changes of this obstructed segment have been performed, the underlying mechanisms are still unclear. The role of extracellular matrix (ECM) components remains controversial, and the investigations in the field of ECM changes, might help the better understanding of the pathogenesis of this common condition. The aim of the present study was to investigate for the first time in the literature whether MMP9 and its specific inhibitors, TIMP1 and RECK, are expressed in a reproducible, specific pattern in UPJ. METHODS: UPJO specimens were obtained from 16 children at the time of dismembered pyeloplasty due to intrinsic UPJ stenosis. Expression levels of the three genes (MMP9, TIMP1 and RECK) were analyzed by quantitative real-time polymerase chain reaction (qRT-PCR). Then correlated the expression levels of the genes according to grade study population that was divided in 2 categories according to Society of Fetal Urology classification, grade 3 (moderate) and 4 (severe). For DTPA we subdivided the childrens in 2 groups, obstructive (T 1/2 more than 20 min) and partial obstructive (T 1/2 between 10 and 20 min) and success in a surgery was defined as decrease in T 1/2 to less than 20 min, absence of symptoms, improving renal function and decreasing dilatation on successive exams. RESULTS: MMP9 was underexpressed and TIMP1 and RECK were overexpressed in children with obstructive DTPA but the differences were not statistically significant. Overexpression of MMP9 was higher among patients with severe grade of UPJ compared to those with moderate grade. Surprisingly expression levels of MMP-9 was three times higher in children who were successfully treated by surgery (n = 10) (p = 0.072), so those who were followed for at least 1 year after surgery and remained with improvement in renal function and decreasing dilation on intravenous urogram and TIMP-1 was underexpressed in 100 % of this cases (p = 0.00). CONCLUSIONS: We showed an increase in expression of MMP9 and a decrease in expression of TIMP1 in children who improving renal function and decreasing dilation after surgery. We believe that the higher expression of MMP9 in these cases can reflect an increase in degradation and remodeling process that could be used as a marker for surgical outcome.

Extreme hydronephrosis due to uretropelvic junction obstruction in infant (case report).

BACKGROUND: Hydronephrosis is the one of the most common congenital abnormalities of urinary tract. The left kidney is more commonly affected than the right side and is more common in males. AIM OF THE STUDY: To determine the role of ultrasonography, renal dynamic scintigraphy and lowerdose computed tomography urography in preoperative diagnostic workup of infant with extreme hydronephrosis. CASE REPORT: We presented the boy with antenatally diagnosed hydronephrosis. In serial, postnatal ultrasonography, renal scintigraphy and computed tomography urography we observed slightly declining function in the dilated kidney and increasing pelvic dilatation. Pyeloplasty was performed at the age of four months with good result. CONCLUSIONS: Results of ultrasonography and renal dynamic scintigraphy in child with extreme hydronephrosis can be difficult to asses, therefore before the surgical procedure a lower-dose computed tomography urography should be performed.

Urinary and serum biomarkers in ureteropelvic junction obstruction: a systematic review.

CONTEXT: Ureteropelvic junction obstruction (UPJO) constitutes a predominant cause of obstructive hydronephrosis. Fundamental questions regarding the assessment and treatment of infants and children with obstructive nephropathy remain unanswered. OBJECTIVE: Several studies have investigated the usefulness of substances that could serve as potential diagnostic and prognostic biomarkers in children with UPJO. Aim of the present study is to systematically review the literature on biomarkers that have been studied to date in patients with UPJO. METHODS: The main search was conducted in the electronic databases MEDLINE and Cochrane Central Register of Controlled Trials (CENTRAL) from inception through March 2014 using various combinations of Medical Subject Headings (MeSH). RESULTS: The 14 included studies reported data on 380 UPJO patients who underwent surgery, 174 who were treated conservatively and 213 controls. CONCLUSION: Some biomarkers offer promising results however more multicenter, prospective carefully designed studies are needed to evaluate their diagnostic and prognostic value.

Double obstruction of ureter: A diagnostic challenge.

INTRODUCTION: Isolated obstruction of the ureteropelvic junction and the vesico-ureteric junction are the two most common causes of hydronephrosis in a pediatric population.[1] They do not pose diagnostic difficulties when are present alone but when together can be difficult to diagnose. Here, we discuss the problems we faced when we encountered these two anomalies in the same ureter and the way in which we managed them. AIM: To assess the difficulties in diagnosis of pediatric patients who present with both ureteropelvic junction obstruction (UPJO) and vesico-ureteric junction obstruction (VUJO) in the ipsilateral ureter and their management protocol. MATERIALS AND METHODS: This is a retrospective study. The study period is from 1 January 2004 to 31 December 2011. Out of 254 children who were diagnosed to have hydronephrosis due to UPJO in our institute, 5 patients (in the age range of 5 to 10 years) had both UPJO and VUJO in the ipsilateral ureter. The problems we faced in diagnosing the two conditions are mentioned with a literature review. RESULTS: Operative intervention was used in four out of five patients; none of the patients had an accurate diagnosis before surgery. All patients were suspected of having double obstruction during pyeloplasty when appropriate size double J stent could not be negotiated through the vesicoureteric junction into the bladder. Postoperative nephrostogram confirmed the diagnosis in all patients. CONCLUSION: Children with double obstruction of the ipsilateral ureter present as a diagnostic dilemma. Because of the rarity of this condition it can escape the eye of even an astute clinician. Early diagnosis can be made if this condition is kept in mind while treating any hydronephrosis due to UPJO or UVJO.

The construction of a nomogram to predict the prognosis and recurrence risks of UPJO.

Objective: This study was conducted to explore the risk factors for the prognosis and recurrence of ureteropelvic junction obstruction (UPJO). Methods: The correlation of these variables with the prognosis and recurrence risks was analyzed by binary and multivariate logistic regression. Besides, a nomogram was constructed based on the multivariate logistic regression calculation. After the model was verified by the C-statistic, the ROC curve was plotted to evaluate the sensitivity of the model. Finally, the decision curve analysis (DCA) was conducted to estimate the clinical benefits and losses of intervention measures under a series of risk thresholds. Results: Preoperative automated peritoneal dialysis (APD), preoperative urinary tract infection (UTI), preoperative renal parenchymal thickness (RPT), Mayo adhesive probability (MAP) score, and surgeon proficiency were the high-risk factors for the prognosis and recurrence of UPJO. In addition, a nomogram was constructed based on the above 5 variables. The area under the curve (AUC) was 0.8831 after self cross-validation, which validated that the specificity of the model was favorable. Conclusion: The column chart constructed by five factors has good predictive ability for the prognosis and recurrence of UPJO, which may provide more reasonable guidance for the clinical diagnosis and treatment of this disease.

Laparoscopic pyeloplasty in neonates and infants is safe and efficient.

Introduction: Dismembered laparoscopic pyeloplasty (LP) is a well-accepted treatment modality for ureteropelvic junction obstruction (UPJO) in children. However, its efficacy and safety in infants, particularly neonates, remain uncertain. To address this significant knowledge gap, we aimed to compare outcomes between a cohort of neonates and infants undergoing LP vs. open pyeloplasty (OP) at less than 6 months and 6 weeks of age. Material and methods: We conducted a retrospective analysis of data from patients who underwent primary pyeloplasty at our institution between 2000 and 2022. Only patients aged 6 months or less at the time of surgery were included, excluding redo-procedures or conversions. Ethical approval was obtained, and data were assessed for redo-pyeloplasty and postoperative complications, classified according to the Clavien-Madadi classification. A standard postoperative assessment was performed 6 weeks postoperatively. This included an isotope scan and a routine ultrasound up to the year 2020. Results: A total of 91 eligible patients were identified, of which 49 underwent LP and 42 underwent OP. Patients receiving LP had a median age of 11.4 (1-25.4) weeks, compared to 13.8 (0.5-25.9) weeks for those receiving OP (p > 0.31). Both groups in our main cohort had an age range of 0-6 months at the time of surgery. Nineteen patients were younger than 6 weeks at the time of surgery. The mean operating time was longer for LP (161 ± 43 min) than that for OP (109 ± 32 min, p < 0.001). However, the mean operating time was not longer in the patient group receiving LP at ≤6 weeks (145 ± 21.6) compared to that in our main cohort receiving LP. There was no significant difference in the length of stay between the groups. Four patients after LP required emergency nephrostomy compared to one patient after OP. The rate of revision pyeloplasty in our main cohort aged 0-6 months at surgery was 8% in the patient group receiving LP and 14% in the patient group receiving OP (not significant). Three revisions after LP were due to persistent UPJO, and one was due to stent migration. Only one patient requiring revision pyeloplasty was less than 6 weeks old. Conclusion: To our knowledge, this is one of the largest collectives of laparoscopic pyeloplasty performed in infants, and it is the youngest cohort published to date. Based on our experience, LP in neonates and infants under 6 months appears to be as effective as open surgery.

Fahr's syndrome as the initial imaging characteristics of MELAS syndrome with a possible seizure activity and cardiac arrest: a case report.

This study reported a case of MELAS syndrome presenting as the initial imaging characteristics of Fahr's syndrome with "near" sudden unexpected death in epilepsy (SUDEP) and lateralized periodic discharges (LPD). The patient, a young boy, experienced loss of consciousness 2 days prior, which was followed by two limb and facial convulsions. He was later found in cardiac arrest during hospitalization, but regained consciousness gradually after receiving cardiopulmonary resuscitation and tracheal intubation. The patient exhibited short stature, intellectual disability, poor sports abilities, and academic performance since childhood, but had no family history. Emergency head computed tomography (CT) revealed high density calcification in bilateral caudate nucleus, lentiform nucleus, thalamus, and dentate nucleus with evidence of an acute process. The patient was transferred to the neurology department where he continued to recover consciousness, though he experienced dysarthria, left limb hemiplegia, and hemiparesthesia. Changes in head magnetic resonance imaging (MRI) findings were noted at admission, 1 month later, and 6 months later. LPD were observed in his video electroencephalogram. The CT urography indicated a narrow left ureteropelvic junction with left hydronephrosis, which was suggestive of ureteropelvic junction obstruction. Ultimately, a diagnosis of near-SUDEP was suspected in this patient, indicating a rare case of MELAS syndrome with near-SUDEP and LPD. The gene tests results revealed the presence of the mitochondrial DNA A3243G mutation, leading to the final diagnosis of MELAS syndrome. This case expands the clinical disease spectrum of the MELAS syndrome.

Case Report; Concomitant Left Nutcracker Syndrome and Right Ureteropelvic Junction Obstruction.

We report rare case of concomitant left nutcracker syndrome and right ureteropelvic junction obstruction (UPJO) on adult female patient diagnosed by CT urogram after she presented with intermittent bilateral flank pain. For this we did Anderson-Hynes pyeloplasty for right ureteropelvic obstruction, it was laparoscopic initially but due to difficulty of stenting it is changed to open. She had smooth post-op course then discharged on 3rd post-op day. Symptoms of nutcracker syndrome are not that much bothersome for the patient so we planned to follow her conservatively.

The learning curve of robot-assisted laparoscopic pyeloplasty in children.

To explore the learning curve of robot-assisted laparoscopic pyeloplasty (RALP) in children. The clinical data, surgical information, and postoperative complications of consecutive cases of RALP performed by the same surgeon in Shanghai Children's Hospital from January 2014 to July 2020 were retrospectively analyzed; the surgeon is a senior pediatric urologists who is proficient in laparoscopic pyeloplasty; the data consist of console time (CT), suture method when anastomosing ureteropelvic junction (UPJ), number of stitches (N), anastomosis time per stitch (tn), the average suture time per stitch (T) = (suture time of first stitch (t1) + second stitch (t2) + … + tn)/N, postoperative complications, and surgical outcome. The learning curve was depicted by cumulative sum method (CUSUM) and validated by cumulative method (CUM). Of the 88 cases, 64 cases were included in present study. Median CT was 104 (83-117) min, mean T was 109 ± 17 s. There were ten cases of Clavien-Dindo Grade I complication and two cases of Clavien-Dindo Grade IIIb complication. The median follow-up time was 237 (87-627) days. The learning curve of CT has three stages, with inflection points at 11th and 57th case, and T has two stages, with inflection points at 19th case. There was a statistically significant difference between the console time and length of stay on both sides of the inflection point (P < 0.05), but there was no statistically significant difference in age, gender, etiology, clinical manifestation, surgical outcome, and complication (P > 0.05). For a senior pediatric urologists who is proficient in laparoscopic pyeloplasty, the learning curve of RALP can be divided into three stages, preliminary exploration stage, mastery stage, and proficiency stage. It takes about 11 cases to achieve the mastery stage, and it takes about 57 cases to achieve the proficiency stage. The learning curve focused on the suture technique of the surgeon is divided into two stages, and after a learning period of 19 cases, it will reach the proficiency stage of suturing UPJ.